Inherited C3 deficiency and meningococcal disease in a teenager.
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Biomedical subjects
Publications and source records attributed to S Schonfeld.
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Over a 34-month period, 1105 newborns weighing between 501 and 2000 g at birth were enrolled in a prospective study of the aetiology and consequences of neonatal brain haemorrhage. The three participating hospitals care for approximately 85% of births in the study weight range in Middlesex, Monmouth and Ocean counties, New Jersey. Cranial ultrasonographic imaging through the anterior fontanelle was carried out a mean age of 4.9 +/- 2.2 hours, 25.5 +/- 4.8 hours and 7.2 +/- 0.8 days to detect haemorrhage and other brain lesions. In 93.2% of study infants, scans were read by two independent expert readers (blind to the clinical status of the child) with submission of the scan to a third reader in cases of disagreement. Confirmation of both presence or absence and, when present, scan of first diagnosis of germinal matrix and/or intraventricular haemorrhage (GM/IVH) by two independent readers was achieved in 76.3% of study infants. The first two readers agreed as to presence or absence of GM/IVH in 82.4% of infants (Kappa = 0.56). Interobserver agreement was affected by the reported scan quality and by the number of scans available, but not by the hospital of origin, race or birthweight of the infant.
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A 25-year-old man, who was admitted for evaluation of arthralgia and fever of 2-weeks duration, complained of a 10 kg weight loss during the previous weeks. Systemic lupus erythematosus (SLE) was diagnosed on the basis of leukopenia, LE cells, antinuclear antibodies, antibodies to double-stranded DNA, and arthritis, Malabsorption was diagnosed because of the finding of hypoalbuminemia, fat droplets in the feces, a pathological D-xylose test, and an appropriate X-ray image. Approximately half the patients with SLE develop minor expressions of gastrointestinal tract involvement, such as nausea, vomiting, and diarrhea. Major manifestations, such as intestinal obstruction or perforation, ascites, peritonitis and pancreatitis have been reported with varying frequency. Despite the frequent association of SLE with gastrointestinal manifestations, malabsorption, as in this case, has rarely been reported.
Patients with systemic lupus erythematosus (SLE) are prone to develop opportunistic infections. Nevertheless, arthritis due to Pseudomonas aeruginosa in SLE is very rare and seems to be related to corticosteroid therapy and previous penetrating injury. A 17-year-old girl with SLE and Pseudomonas arthritis and discitis which followed laparotomy is described. Arthritis is a common manifestation of SLE, but arthritis due to infection with Pseudomonas is very serious and the diagnosis should not be missed.
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Chronic subdural hematoma presenting as transient ischemic attacks (TIA) is very rare. In a 74-year-old man in whom chronic subdural hematoma was responsible for recurrent attacks of local neurological deficiencies, the diagnosis was established by computerized tomographic (CT) scanning. Both TIA and chronic subdural hematoma usually occur in the elderly. Since the prognosis as well as the therapeutic approach are completely different in the 2 conditions, it is advisable to use imaging techniques, such as CT scanning, when there are recurrent TIA.
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The reliability of cranial ultrasound diagnosis in the premature neonate was examined using data from an ongoing multicentre study of the epidemiology and long-term consequences of neonatal brain haemorrhage. First week ultrasound films (obtained at 4 hours, 24 hours and 7 days) from 60 study subjects were randomly selected for independent review by two groups of experienced interpreters, and results were recorded separately for observations (i.e. presence or absence of an abnormal echodense area on a film) and interpretations (i.e. presence or absence of haemorrhage or ventricular dilatation) in each hemisphere. Because of deaths in the first week of life, the total number of films examined was 138. Concordance on the presence or absence of an abnormal echodensity was examined for each individual film for three areas of interest: the germinal matrix, the ventricles and the parenchyma. Concordance on the presence or absence of haemorrhage or ventricular dilatation was examined only for the seventh-day film, or the final film prior to death. Finally, concordance was analysed with the diagnostic interpretations grouped into categories thought to differ prognostically for long-term outcome. In general, concordance was poorest for germinal matrix lesions and best for parenchymal lesions. Concordance was lower for observations made on each individual film than it was for interpretation of the final film in each case. Fifty-five of 60 cases (92%) were assigned to the same major prognostic category by both readers. Ultrasound review conferences were held periodically and there was evidence that concordance in ultrasound reading and interpretation improved during the course of the study.
Angioimmunoblastic lymphadenopathy with dysproteinemia is a recently described entity with severe clinical symptoms and characteristic histological findings in the lymph nodes with occasional malignant transformation. Kaposi's sarcoma is a neoplastic angiomatous growth of unknown origin. Alterations in the immune system have been reported in both diseases. The association of angioimmunoblastic lymphadenopathy with dysproteinemia and Kaposi's sarcoma in the same patient may suggest a common origin for both.
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Eosinophilic granulomas of the stomach, also known as inflammatory pseudotumours, are infrequently occurring lesions characterised histologically by local capillary and fibroblastic proliferation with infiltration of eosinophilic cells. This condition is benign and appears as a polypoid mass with or without ulceration. A case of association of a polyarthritis with a eosinophilic polypoid gastric granuloma is described. This was subsequently removed by endoscopic polypectomy.
The identification of two patients who were misusing meperidine prescribed in a hospital prompted the establishment of a surveillance system to examine the possible extent of the problem of prescription narcotic abuse. All outpatient prescriptions for meperidine in a six-month period were reviewed and 17 high users were identified who were considered to be at greatest risk of misuse; they received 55% of all meperidine dispensed from the OPD pharmacy. These persons obtained their prescriptions primarily through the emergency room, they rarely saw the same physician twice, and 12 of them carried a diagnosis of sickle cell disease. Among these patients, four cases of definite abuse were identified. Interventions aimed at promoting continuity of care to the high users led to a 66% reduction in meperidine prescribed to this group in the emergency room and to a shift in their care to the clinics. Establishment of a registry for narcotic surveillance is a first toward recognition of outpatient drug abuse. Limiting prescribing privileges to the physician responsible for a patient's long-term care can further decrease this risk.
The association between hyperthyroidism and thrombocytopenia is a known although infrequent clinical condition. Distinct mechanisms are probably active in each particular case, but the thyrotoxic state has been implicated as having a key effect on the fall in the number of platelets. We describe a patient with coexisting Graves' disease and idiopathic thrombocytopenic purpura who showed special refractoriness to treatment of the bleeding condition in the thyrotoxic state, but who promptly responded to treatment when the thrombocytopenia relapsed 2 1/2 years later, while he was euthyroid. Thus, in this case, a clear exacerbating effect of the thyrotoxic state on the thrombocytopenia was observed. We suggest evaluation of the thyroid condition in patients suffering from refractory thrombocytopenia.